Pulmonary hypertension Cause & Symptom

Pulmonary hypertension Cause & Symptom - Definition - Pulmonary hypertension is a type of high blood pressure that affects the arteries in the lungs and the right side of your heart. 
Pulmonary hypertension begins when tiny arteries in the lungs, called pulmonary arteries, and capillaries become narrowed, blocked or damaged. This makes it difficult for blood to flow through the lungs, which increases the pressure in the arteries in your lungs. When the pressure increases, the lower right chamber of the heart (the right ventricle) must work harder to pump blood through the lungs, causing the heart muscle to weaken and ultimately does not work. 
Pulmonary hypertension is a serious disease that worsens and sometimes fatal. Although pulmonary hypertension is not curable, treatments are available to help reduce symptoms and improve the quality of your life. 

The signs and symptoms of pulmonary hypertension at an early stage may not be seen for months or even years. The course of the disease, the symptoms become worse. 

Symptoms of pulmonary hypertension include: 

  • Shortness of breath (dyspnea), initially on exertion and eventually at rest 
  • Fatigue 
  • Dizziness or fainting (syncope) 
  • Or chest pain 
  • Swelling (edema) in the leg and finally the abdomen (ascites) 
  • Bluish color of the lips and skin (cyanosis) 
  • Rapid pulse or heart palpitations 

The heart has two rooms upstairs and two rooms downstairs. Every moment of blood through the heart, the lower right chamber (right ventricle) pumps blood to the lungs through a large blood vessel (pulmonary artery). In the lungs, the blood releases carbon dioxide and takes on oxygen. Oxygen-rich blood then flows through the blood vessels in the lungs (arteries, capillaries and pulmonary veins) to the left side of the heart. 
Normally, blood flows easily through the vessels in the lungs, so blood pressure is usually much lower in the lungs. On pulmonary hypertension, rise in blood pressure caused by changes in the cells lining the pulmonary artery. These changes lead to additional tissue is formed, eventually narrowing or completely blocking the blood vessels, making the blood vessels stiff and narrow. This makes it harder for blood to flow, increases blood pressure in the pulmonary artery. 

When the underlying cause for pulmonary hypertension can not be found, the condition is called idiopathic pulmonary hypertension (Idiopathic Pulmonary Hypertension / IPH). 
Some people with IPH may have genes that are risk factors for developing pulmonary hypertension. But in most people with IPH, no definite cause. 

Secondary Pulmonary Hypertension 
Pulmonary hypertension caused by another medical problem is called secondary pulmonary hypertension. Types of pulmonary hypertension is more common than idiopathic pulmonary hypertension. Causes of secondary pulmonary hypertension include: 

  • Blood clots in the lungs (pulmonary embolism) 
  • Chronic obstructive pulmonary disease, such as emphysema 
  • Connective tissue disorders such as scleroderma or lupus 
  • Sleep apnea and other sleep disorders 
  • Congenital heart disease 
  • Sickle cell anemia 
  • Chronic liver disease (cirrhosis) 
  • AIDS 
  • Lung diseases such as pulmonary fibrosis, a condition that causes scarring in the tissue between the air sacs of the lungs (interstitium) 
  • Left heart failure 
  • Live at a higher altitude than 8,000 feet (2,438 meters) 
  • Rock climbing or hiking to altitudes higher than 8,000 feet (2,438 meters) without adjustment first. 
  • The use of certain drugs, such as cocaine 

Risk Factors 
Although anyone can be affected by both types of pulmonary hypertension, older people tend to suffer from secondary pulmonary hypertension, and younger people tend to suffer from idiopathic pulmonary hypertension. Idiopathic pulmonary hypertension is also more common in women than men. 
Another risk factor for pulmonary hypertension is a family history of disease. Some genes may be associated with idiopathic pulmonary hypertension. These genes can cause an overgrowth of cells in the small arteries of your lungs, making it more narrow. 
If one of your family members suffers from idiopathic pulmonary hypertension and showed a positive test for gene mutations that can lead to pulmonary hypertension, your doctor or a genetic counselor may recommend that you or your family members will test the gene mutation. 

Pulmonary hypertension can cause a number of complications, including: 

Right heart failure (Cor pulmonale). 
Blood clots. 
Arrhythmia (irregular heartbeat). 

Test & Diagnosis 

Transesophageal echocardiogram. 
Right heart catheterization. 

Additional tests: 

Pulmonary function tests. 
Perfusion lung scan. 
Computerized tomography (CT) scan. 
Magnetic Resonance Imaging (MRI). 
Lung biopsy. 
Genetic tests 

Classification of Pulmonary Hypertension 
After being diagnosed with pulmonary hypertension, your doctor may classify disease using guidelines developed by the New York Heart Association. 

Class I. Although you have been diagnosed with pulmonary hypertension, you do not have symptoms. 
Class II. You do not have symptoms at rest, but you have fatigue, shortness of breath or chest pain with normal activity. 
Class III. You feel comfortable at rest but have symptoms when you are physically active. 
Class IV. You have symptoms even at rest. 


Endotelin.Reseptor antagonist 
High doses of calcium channel blockers. 

• Atrium septostomy. If the medication can not control your pulmonary hypertension, open heart surgery may be an option. In atrial septostomy, the surgeon will create a gap between the left and right chamber of your heart to relieve the pressure on the right side of your heart. Atrial septostomy can have serious complications, including heart rhythm abnormalities (arrhythmias). 
• Transplantation. In some cases, lung transplantation or heart-lung might be an option, especially for young people who have idiopathic pulmonary hypertension. The main risk of any type of transplants, including organ transplant rejection and serious infection, and you have to take immunosuppressant drugs for life to help reduce the possibility of rejection.